Saturday, November 12, 2016

Maine Medical Center received confirmation patient treated at the hospital has Creutzfeldt-Jakob disease

Date:  Nov. 11, 2016 Contact: Clay Holtzman Communications & Public Affairs  

Maine Medical Center receives confirmation that patient had rare brain disorder

PORTLAND, Maine - Maine Medical Center today received confirmation that a patient treated at the hospital has Creutzfeldt-Jakob disease, a degenerative brain disorder caused by an infectious type of protein.

Hospital officials learned earlier in the week that a patient appeared to have the rare condition based on an initial biopsy result. On Friday the National Prion Disease Pathology Surveillance Center at Case Western University provided confirmation of the diagnosis.

Transmission of Creutzfeldt-Jakob disease in a hospital setting is extremely rare, with no confirmed incidence of such transmission in more than 20 years. Nevertheless the hospital took extraordinary steps to assure patient safety. Approximately 150 elective surgery cases were rescheduled Wednesday and Thursday while the hospital decontaminated surgical equipment and facilities in accordance with guidelines set by the U.S. Centers for Disease Control and Prevention.

"We thought it important to go above and beyond to assure the safety of our patients," said Joel Botler, M.D., chief medical officer at MMC. While the hospital does track surgical equipment used in each case, the decision was made not to rely on tracking and instead make sure that any piece of equipment that could potentially be affected was treated. "Now that we know this case is confirmed, we can see that our

response was 100 percent appropriate and that patients should feel confident in the safety of their care at Maine Medical Center," added Botler.

The hospital is now looking at the types of procedures performed and instruments used to determine which patients should be specifically contacted because they were treated prior to the initial pathology report showing a patient had Creutzfeldt-Jakob disease.

"We are in the process of reaching out to the small number of patients who we think should be notified based on the details of their specific case," said Botler. "Our staff members have been fielding calls from patients who have legitimate concerns and questions about their care. Let me be clear, only a small number of patients who have had surgery at MMC have been exposed to any degree of risk, and that risk is exceedingly low approaching zero."

Botler said more than 100 MMC employees worked around the clock to decontaminate equipment. In the meantime, other Maine hospitals loaned equipment so that MMC could perform the emergency surgeries that are required of the state's largest medical center and Maine's only Level 1 Trauma Center.   "We want to thank our amazing team here at Maine Medical Center," said Botler. "This is an especially rare circumstance in medicine, and the men and women here responded with the utmost professionalism and dedication to make sure we continue to provide our patients with excellent, safe patient-centered care. We want to also thank our patients for their understanding and apologize for any inconvenience and concern this has caused them and their families. Finally we’d like to thank all of our colleagues in the statewide healthcare community who have come to our assistance."

About Maine Medical Center Maine Medical Center (MMC), recognized as the number-one ranked hospital in Maine by U.S. News and World Report for 2016-2017, is a complete health care resource for the people of Greater Portland and the entire state, as well as northern New England. Incorporated in 1864, MMC is the state’s largest medical center, licensed for 637 beds and employing nearly 6,500 people. MMC's unique role as both a community hospital and a referral center requires an unparalleled depth and breadth of services, including an active educational program and a world-class biomedical research center. As a nonprofit institution, Maine Medical Center provides nearly 23 percent of all the charity care delivered in Maine. MMC is a member of the MaineHealth system, a growing family of health care services in northern New England. For more information, visit

Wednesday, November 09, 2016

Maine Medical Center postpones elective surgeries over suspected case of prion disease

Friday, November 11, 2016

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

kind regards, terry

Friday, November 11, 2016

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

·         David J. Bonda, MD1, 

·         Sunil Manjila, MD1, 

·         Prachi Mehndiratta, MD2, 

·         Fahd Khan, MD3, 

·         Benjamin R. Miller,MD1, 

·         Kaine Onwuzulike, MD1, 

·         Mark L. Cohen, MD5,6, 

·         Ignazio Cali, PhD4,6

1Department of Neurological Surgery, University Hospitals Case Medical Center, and 5National Prion Disease Pathology Surveillance Center, 6Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio; 2Department of Neurology, University of Virginia Health System, Charlottesville, Virginia; 3Department of Neurosurgery, Stanford University, Stanford, California; 4Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy; and 7Division of High-Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia

ABBREVIATIONS BSE = bovine spongiform encephalopathy; CDC = Centers for Disease Control and Prevention; CJD = Creutzfeldt-Jakob disease; dCJD = CJD transmitted by commercially distributed cadaveric dura mater; EEG = electroencephalography; fCJD = familial CJD; FFI = fatal familial insomnia; GSS = Gerstmann-Schäussler-Scheinker syndrome; hGH = human growth hormone; iCJD = iatrogenic CJD: M = methionine; NIH = National Institutes of Health; PrP = prion protein; PrPC = cellular PrP; PrPSc = abnormal form of PrP; sCJD = sporadic CJD; sFI = sporadic fatal insomnia; V = valine;vCJD = variant CJD; VPSPr = variably protease-sensitive prionopathy; WHO = World Health Organization.

INCLUDE WHEN CITING DOI: 10.3171/2016.5.FOCUS15126.

Correspondence Ignazio Cali, Department of Pathology, Case Western Reserve University, 2085 Adelbert Rd., Cleveland, OH 44106. email:

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The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood “infectious protein” has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts. Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern. Laboratory studies indicate that standard decontamination and sterilization procedures may be insufficient to completely remove infectivity from prion-contaminated instruments. In this unfortunate event, the instruments may transmit the prion disease to others. Much caution therefore should be taken in the absence of strong evidence against the presence of a prion disease in a neurosurgical patient. While the Centers for Disease Control and Prevention (CDC) and World Health Organization (WHO) have devised risk assessment and decontamination protocols for the prevention of iatrogenic transmission of the prion diseases, incidents of possible exposure to prions have unfortunately occurred in the United States. In this article, the authors outline the historical discoveries that led from kuru to the identification and isolation of the pathological prion proteins in addition to providing a brief description of human prion diseases and iatrogenic forms of CJD, a brief history of prion disease nosocomial transmission, and a summary of the CDC and WHO guidelines for prevention of prion disease transmission and decontamination of prion-contaminated neurosurgical instruments.

Wednesday, November 09, 2016

Maine Medical Center postpones elective surgeries over suspected case of prion disease

Wednesday, November 09, 2016

Maine Medical Center postpones elective surgeries over suspected case of prion disease

Sunday, November 06, 2016

UK Iatrogenic Creutzfeldt–Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches

Thursday, April 12, 2012

*** Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010

Thursday, August 13, 2015

Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years

Friday, January 10, 2014

vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

Greetings Friends, Neighbors, and Colleagues,

Wednesday, September 07, 2016

Michigan Launches an investigation into the Detroit Medical Center dirty, broken and missing surgical instruments, what about the CJD TSE PRION iatrogenic threat past and present therefrom?

Thursday, January 14, 2016

*** Preventable Tragedies: Superbugs and How Ineffective Monitoring of Medical Device Safety Fails Patients REPORT ***

how can it be, HOW CAN IT BE $$$ not a word about CJD GSS FFI VPSPR TSE Prions that I saw...absolutely crazy, WE ARE MISSING THE BIGGER PICTURE!

how many victims that will never be reported ???

Sunday, January 17, 2016

*** Of Grave Concern Heidenhain Variant Creutzfeldt Jakob Disease ***

Thursday, June 04, 2015

Catholic Medical Center v. Civil No. 14-cv-180-JL Opinion No. 2015 DNH 110 Fireman’s Fund Insurance Company Creutzfeldt Jakob Disease TSE Prion tainted medical instruments


Tuesday, February 11, 2014

Novant Health Forsyth Medical Center Information on potential CJD exposure

Monday, February 10, 2014

18 Forsyth Medical Center patients exposed to CJD; apology issued...OOOPS, SORRY, TOO BAD $$$

Thursday, January 16, 2014

The Anspach Effort, Inc. RECALL FDA Blackmax motor had been used in a case where the patient was diagnosed with Creutzfeldt-Jacob Disease (CJD) MARYLAND HOSTPITAL

Friday, January 10, 2014

*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ??? ***

Thursday, January 23, 2014

Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]

Sunday, April 06, 2014

SPORADIC CJD and the potential for zoonotic transmission there from, either directly or indirectly via friendly fire iatrogenic mode, evidence to date

Thursday, April 17, 2014

Novant: Three more may have been exposed to disease CJD

Wednesday, September 10, 2014

Creutzfeldt-Jakob disease (CJD) biannual update (August 2014), with updated guidance on decontamination of gastrointestinal endoscopy equipment

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Tuesday, August 26, 2014

Blood reference materials from macaques infected with variant Creutzfeldt-Jakob disease agent

Wednesday, November 27, 2013

NHS failed to sterilise surgical instruments contaminated with 'mad cow' disease

Saturday, November 16, 2013

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

Infect Control Hosp Epidemiol.

Tuesday, September 24, 2013

NORDION (US), INC., AND BIOAXONE BIOSCIENCES, INC., Settles $90M Mad Cow TSE prion Contamination Suit Cethrin(R)

Case 0:12-cv-60739-RNS Document 1 Entered on FLSD Docket 04/26/2012 Page 1 of 15

Thursday, September 05, 2013

Possible Patient Exposure to Creutzfeldt-Jakob Disease Announced New Hampshire DHHS

Press Release

Friday, July 19, 2013

Beaumont Hospital in Dublin assessing patients for CJD

Monday, April 15, 2013

Dr. Stephen B. Thacker Director Centers for Disease Control and Prevention′s Office of Science, Epidemiology and Laboratory Services (OSELS) dies from Creutzfeldt Jakob Disease CJD

Thursday, April 12, 2012

Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010

Eurosurveillance, Volume 17, Issue 15, 12 April 2012

Research articles

Tuesday, July 31, 2012

11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital

Thursday, August 02, 2012

CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients

Tuesday, July 31, 2012

11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital

Thursday, August 02, 2012

CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients

Saturday, February 12, 2011

Another Pathologists dies from CJD, another potential occupational death ?

another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???

Wednesday, November 30, 2011

First iCJD Death Confirmed in Korea

Thursday, December 08, 2011

A case of Iatrogenic Creutzfeldt Jakob Disease (iCJD) in a patient who had received a German-manufactured human dura mater graft 23 years ago

Thursday, December 8, 2011

S. Korea confirms second case of iatrogenic Creutzfeldt-Jakob disease 48-year-old man

2011/12/08 11:08 KST

Saturday, February 13, 2016

The Risk of Prion Infection through Bovine Grafting Materials in dentistry

Saturday, January 16, 2016

Revised Preventive Measures to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease and Variant Creutzfeldt-Jakob Disease by Blood and Blood Products Guidance for Industry

Tuesday, May 26, 2015

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Friday, October 09, 2015

*** An alarming presentation level II trauma center of Creutzfeldt-Jakob disease following a self-inflicted gunshot wound to the head

Wednesday, January 06, 2016


Thursday, March 17, 2016

Preliminary Diagnosis Creutzfeldt-Jakob Disease Confirmed in Patient that had Lumbar Puncture at Washington Regional Medical Center

all iatrogenic cjd is, is sporadic cjd, until the iatrogenic event is discovered, traced back, documented in the Academic domain, and then put into the public domain and documented as an iatrogenic CJD event. that’s why 85%+ of all human TSE prion disease is still sporadic CJD. problem solved $$$


Thursday, September 10, 2015

25th Meeting of the Transmissible Spongiform Encephalopathies Advisory Committee Food and Drug Administration Silver Spring, Maryland June 1, 2015

Saturday, February 13, 2016

The Risk of Prion Infection through Bovine Grafting Materials in dentistry

Monday, February 15, 2016

Distinctive properties of plaque-type dura mater graft-associated Creutzfeldt–Jakob disease in cell-protein misfolding cyclic amplification

I would kindly like to comment and try to bring awareness, NOT to any potential cure, for this would be great, but what about cause???

the scientific literature has been supporting prion-like transmission for Alzheimer’s for decades, the world chose to ignore it, thus not fund for further said transmission studies, and there are only two scenarios I see, and none have been fun-fact finding for me.

scenario one, if we wait any longer, we have the potential to lose a whole generation of humans to Alzheimer’s via iatrogenic mode if something is not done to protect humans and animals from certain medical procedures.

scenario two, it may already be too late. ...

Terry S. Singeltary Sr.

Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy

07 02:27 AM

Terry S. Singeltary Sr. said:

re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy

2015-12-07 02:27 AM

Terry S. Singeltary Sr. said:

re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy

Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)

I would kindly like to comment on the Nature Paper, the Lancet reply, and the newspaper articles.

First, I applaud Nature, the Scientist and Authors of the Nature paper, for bringing this important finding to the attention of the public domain, and the media for printing said findings.

Secondly, it seems once again, politics is getting in the way possibly of more important Transmissible Spongiform Encephalopathy TSE Prion scientific findings. findings that could have great implications for human health, and great implications for the medical surgical arena. but apparently, the government peer review process, of the peer review science, tries to intervene again to water down said disturbing findings.

where have we all heard this before? it’s been well documented via the BSE Inquiry. have they not learned a lesson from the last time?

we have seen this time and time again in England (and other Country’s) with the BSE mad cow TSE Prion debacle.

That ‘anonymous' Lancet editorial was disgraceful. The editor, Dick Horton is not a scientist.

The pituitary cadavers were very likely elderly and among them some were on their way to CJD or Alzheimer's. Not a bit unusual. Then the recipients who got pooled extracts injected from thousands of cadavers were 100% certain to have been injected with both seeds. No surprise that they got both diseases going after thirty year incubations.

That the UK has a "system in place to assist science journalists" to squash embargoed science reports they find ‘alarming’ is pathetic.

Sounds like the journalists had it right in the first place: ‘Alzheimer’s may be a transmissible infection’ in The Independent to ’You can catch Alzheimer’s’ in The Daily Mirror or ‘Alzheimer’s bombshell" in The Daily Express.

if not for the journalist, the layperson would not know about these important findings.

where would we be today with sound science, from where we were 30 years ago, if not for the cloak of secrecy and save the industry at all cost mentality?

when you have a peer review system for science, from which a government constantly circumvents, then you have a problem with science, and humans die.

to date, as far as documented body bag count, with all TSE prion named to date, that count is still relatively low (one was too many in my case, Mom hvCJD), however that changes drastically once the TSE Prion link is made with Alzheimer’s, the price of poker goes up drastically.

so, who makes that final decision, and how many more decades do we have to wait?

the iatrogenic mode of transmission of TSE prion, the many routes there from, load factor, threshold from said load factor to sub-clinical disease, to clinical disease, to death, much time is there to spread a TSE Prion to anywhere, but whom, by whom, and when, do we make that final decision to do something about it globally? how many documented body bags does it take? how many more decades do we wait? how many names can we make up for one disease, TSE prion?

Professor Collinge et al, and others, have had troubles in the past with the Government meddling in scientific findings, that might in some way involve industry, never mind human and or animal health.

FOR any government to continue to circumvent science for monetary gain, fear factor, or any reason, shame, shame on you.

in my opinion, it’s one of the reasons we are at where we are at to date, with regards to the TSE Prion disease science i.e. money, industry, politics, then comes science, in that order.

greed, corporate, lobbyist there from, and government, must be removed from the peer review process of sound science, it’s bad enough having them in the pharmaceutical aspect of healthcare policy making, in my opinion.

my mother died from confirmed hvCJD, and her brother (my uncle) Alzheimer’s of some type (no autopsy?). just made a promise, never forget, and never let them forget, before I do.

I kindly wish to remind the public of the past, and a possible future we all hopes never happens again. ...

[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. There are also results to be made available shortly (1) concerning a farmer with CJD who had BSE animals, (2) on the possible transmissibility of Alzheimer’s and (3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]

snip...see full Singeltary Nature comment here;

see Singeltary comments to Plos ;


BSE101/1 0136



From: . Dr J S Metiers DCMO

4 November 1992


1. Thank you for showing me Diana Dunstan's letter. I am glad that MRC have recognised the public sensitivity of these findings and intend to report them in their proper context. 'This hopefully will avoid misunderstanding and possible distortion by the media to portray the results as having more greater significance than the findings so far justify.

2. Using a highly unusual route of transmission (intra-cerebral injection) the researchers have demonstrated the transmission of a pathological process from two cases one of severe Alzheimer's disease the other of Gerstmann-Straussler disease to marmosets. However they have not demonstrated the transmission of either clinical condition as the "animals were behaving normally when killed". As the report emphasises the unanswered question is whether the disease condition would have revealed itself if the marmosets had lived longer. They are planning further research to see if the conditions, as opposed to the partial pathological process, is transmissible.

what are the implications for public health?

3. The route 'of transmission is very specific and in the natural state of things highly unusual. However it could be argued that the results reveal a potential risk, in that brain tissue from these two patients has been shown to transmit a pathological process. Should therefore brain tissue from such cases be regarded as potentially infective? Pathologists, morticians, neuro surgeons and those assisting at neuro surgical procedures and others coming into contact with "raw" human brain tissue could in theory be at risk. However, on a priori grounds given the highly specific route of transmission in these experiments that risk must be negligible if the usual precautions for handling brain tissue are observed.



BSE101/1 0137

4. The other dimension to consider is the public reaction. To some extent the GSS case demonstrates little more than the transmission of BSE to a pig by intra-cerebral injection. If other prion diseases can be transmitted in this way it is little surprise that some pathological findings observed in GSS were also transmissible to a marmoset. But the transmission of features of Alzheimer's pathology is a different matter, given the much greater frequency of this disease and raises the unanswered question whether some cases are the result of a transmissible prion. The only tenable public line will be that "more research is required’’ before that hypothesis could be evaluated. The possibility on a transmissible prion remains open. In the meantime MRC needs carefully to consider the range and sequence of studies needed to follow through from the preliminary observations in these two cases. Not a particularly comfortable message, but until we know more about the causation of Alzheimer's disease the total reassurance is not practical.

J S METTERS Room 509 Richmond House Pager No: 081-884 3344 Callsign: DOH 832 llllYc!eS 2 92/11.4/1.2

>>> The only tenable public line will be that "more research is required’’ <<<

>>> possibility on a transmissible prion remains open<<<

O.K., so it’s about 23 years later, so somebody please tell me, when is "more research is required’’ enough time for evaluation ?

Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease

*** Singeltary comment PLoS ***

Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?

Posted by flounder on 05 Nov 2014 at 21:27 GMT

Sunday, November 22, 2015

*** Effect of heating on the stability of amyloid A (AA) fibrils and the intra- and cross-species transmission of AA amyloidosis Abstract

Amyloid A (AA) amyloidosis is a protein misfolding disease characterized by extracellular deposition of AA fibrils. AA fibrils are found in several tissues from food animals with AA amyloidosis. For hygienic purposes, heating is widely used to inactivate microbes in food, but it is uncertain whether heating is sufficient to inactivate AA fibrils and prevent intra- or cross-species transmission. We examined the effect of heating (at 60 °C or 100 °C) and autoclaving (at 121 °C or 135 °C) on murine and bovine AA fibrils using Western blot analysis, transmission electron microscopy (TEM), and mouse model transmission experiments. TEM revealed that a mixture of AA fibrils and amorphous aggregates appeared after heating at 100 °C, whereas autoclaving at 135 °C produced large amorphous aggregates. AA fibrils retained antigen specificity in Western blot analysis when heated at 100 °C or autoclaved at 121 °C, but not when autoclaved at 135 °C. Transmissible pathogenicity of murine and bovine AA fibrils subjected to heating (at 60 °C or 100 °C) was significantly stimulated and resulted in amyloid deposition in mice. Autoclaving of murine AA fibrils at 121 °C or 135 °C significantly decreased amyloid deposition. Moreover, amyloid deposition in mice injected with murine AA fibrils was more severe than that in mice injected with bovine AA fibrils. Bovine AA fibrils autoclaved at 121 °C or 135 °C did not induce amyloid deposition in mice. These results suggest that AA fibrils are relatively heat stable and that similar to prions, autoclaving at 135 °C is required to destroy the pathogenicity of AA fibrils. These findings may contribute to the prevention of AA fibril transmission through food materials to different animals and especially to humans.

Purchase options Price * Issue Purchase USD 511.00 Article Purchase USD 54.00

*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

Saturday, April 23, 2016


Saturday, April 23, 2016

SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online

Taylor & Francis

Prion 2016 Animal Prion Disease Workshop Abstracts

WS-01: Prion diseases in animals and zoonotic potential

Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,

Natalia Fernandez-Borges a. and Alba Marin-Moreno a

"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France

Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.

To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.

These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.

Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

why do we not want to do TSE transmission studies on chimpanzees $

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.



*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.

*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.

*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France

Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,

***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),

***is the third potentially zoonotic PD (with BSE and L-type BSE),

***thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.


***thus questioning the origin of human sporadic cases***


***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.


SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online

Monday, May 02, 2016

*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***

Saturday, February 6, 2016

*** Secretary's Advisory Committee on Animal Health; Meeting [Docket No. APHIS-2016-0007] Singeltary Submission ***

Saturday, April 16, 2016

*** APHIS [Docket No. APHIS-2016-0029] Secretary's Advisory Committee on Animal Health; Meeting May 2, 2016, and June 16, 2016 Singeltary Submission ***

Transmissible Spongiform Encephalopathy TSE PRION UPDATE

Saturday, July 23, 2016


Tuesday, July 26, 2016

Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016

Monday, August 22, 2016